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Gasdermin E (GSDME), a member of the gasdermin protein family, is associated with post-lingual hearing loss. All GSDME pathogenic mutations lead to skipping exon 8; however, the molecular mechanisms underlying hearing loss caused by GSDME mutants remain unclear. GSDME was recently identified as one of the mediators of programmed cell death, including apoptosis and pyroptosis. Therefore, in this study, we injected mice with GSDME mutant (MT) and examined the expression levels to assess its effect on hearing impairment. We observed loss of hair cells in the organ of Corti and spiral ganglion neurons. Further, the N-terminal release from the GSDME mutant in HEI-OC1 cells caused pyroptosis, characterized by cell swelling and rupture of the plasma membrane, releasing lactate dehydrogenase and cytokines such as interleukin-1ß. We also observed that the N-terminal release from GSDME mutants could permeabilize the mitochondrial membrane, releasing cytochromes and activating the mitochondrial apoptotic pathway, thereby generating possible positive feedback on the cleavage of GSDME. Furthermore, we found that treatment with disulfiram or dimethyl fumarate might inhibit pyroptosis and apoptosis by inhibiting the release of GSDME-N from GSDME mutants. In conclusion, this study elucidated the molecular mechanism associated with hearing loss caused by GSDME gene mutations, offering novel insights for potential treatment strategies.
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Apoptosis , Piroptosis , Piroptosis/genética , Animales , Ratones , Mutación con Ganancia de Función , Pérdida Auditiva/genética , Pérdida Auditiva/patología , Humanos , Ganglio Espiral de la Cóclea/metabolismo , Ganglio Espiral de la Cóclea/patología , Órgano Espiral/metabolismo , Órgano Espiral/patología , Células Ciliadas Auditivas/metabolismo , Células Ciliadas Auditivas/patología , GasderminasRESUMEN
Background: Meniere disease, characterized by intermittent episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural pressure, is a common cause of vertigo in humans. The pathogenesis of Meniere disease remains unknown. The current study aimed to describe a novel pathological change discovered in the inner ears of patients with Meniere disease who underwent labyrinthectomy. Methods: This retrospective case-control study was conducted with 21 patients with MD who underwent labyrinthectomy. A total of 15 patients diagnosed with acoustic neuroma or glomus jugular tumor were review over the same period of time as control. The clinical information of the patients and the pathological features of the membrane are described. Results: The new pathological tissue was a morbid membrane structure sealing the round window, characterized by the formation of lymphatic capillaries. Histochemical and immunofluorescent staining was positive for D2-40, LYVE-1, podoplanin, and PROX1, which are the classical markers of the lymphatic vessels. Transmission electron microscopy revealed that the lymph capillaries lacked a typical basement membrane and that their ends were blind, composed of a single layer of endothelial cells with valval connection structures between adjacent capillary epithelial cells. Conclusion: This is the first report of lymphatic vessels in the human inner ear, and this pathological structure is a completely new discovery. The lymphatic vessels may develop due to inflammation or decompensation of pressure in the inner ear, suggesting that the inner ear can reactively form lymphatic vessels in some inflammation and fluid flow-dependent pathological conditions. The current findings help in improving our understanding of the pathogenesis of Meniere disease.
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Objective:To summarize the clinical characteristics and therapeutic effect of traumatic facial nerve palsy. Methods:Sixty-eight cases of traumatic facial nerve palsy were retrospectively analyzed from January 2015 to May 2023. Results:The median course of disease was 33 days. The facial nerve function of the patients was grade HB-â ¡in 2 cases, grade HB-â ¢ in 4 cases, grade HB-â £in 16 cases, grade HB-â ¤ in 37 casesï¼38 earsï¼, and grade HB-â ¥ in 9 cases. 42 cases occurred immediately after injury and 26 cases were delayed. CT examination of temporal bone revealed longitudinal fractures in 51 casesï¼52 earsï¼ , transverse fractures in 6 cases and mixed fractures in 4 cases. No definite temporal bone fracture was found in the remaining 7 cases. The segments of facial nerve injury in 49 casesï¼50 earsï¼ were geniculate ganglion and adjacent, in 7 cases were vertical segment, in 7 cases were horizontal segment, in 2 cases were horizontal segment and vertical segment; and the other 3 cases could not be evaluated. Conservative treatment with glucocorticoids was used in 23 ears and surgery was used in 46 ears. Patients were followed up 6-24 months after treatment, including 20 cases of grade HB-â , 19 cases of grade HB-â ¡, 23 casesï¼24 earsï¼ of grade HB-â ¢, 4 cases of grade HB-â £, and 1 case of grade HB-â ¤.One patient was lost to follow-up. After treatment, the facial nerve function of patients was significantly improvedï¼P<0.05ï¼, and there were significant differences between conservative treatment group and surgical treatment group in the course of facial nerve palsy, the ratio of facial palsy immediately after injury, the nerve function before treatment and the nerve function after treatmentï¼P<0.05ï¼. There were no significant differences in age, sex, hearing condition, temporal bone fracture, facial nerve injury segment and rate of favorable neurologic outcomesï¼P>0.05ï¼. The comparison of patients with neurodegeneration rate>90% and ≤90% showed that the facial nerve function of patients with neurodegeneration rate>90% before treatment was significantly worseï¼P<0.05ï¼, but there was no significant difference between the facial nerve function after treatmentï¼P>0.05ï¼. There was no significant difference in facial nerve function between middle fossa approach group and mastoid approach groupï¼P>0.05ï¼. Conclusion:Patients with traumatic facial nerve palsy should be evaluated individually. Patients with mild facial nerve palsy, low neurodegeneration rate and short course of disease can be treated conservatively and followed up closely. Patients with severe facial nerve palsy, high neurodegeneration rate and more than 6 weeks of disease can be actively considered surgery. Good prognosis can be obtained by correct evaluation and treatment.
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Traumatismos del Nervio Facial , Parálisis Facial , Humanos , Parálisis Facial/etiología , Parálisis Facial/diagnóstico , Parálisis Facial/terapia , Estudios Retrospectivos , Masculino , Femenino , Traumatismos del Nervio Facial/terapia , Traumatismos del Nervio Facial/diagnóstico , Adulto , Persona de Mediana Edad , Adulto Joven , Adolescente , Hueso Temporal/lesiones , Nervio Facial , Glucocorticoides/uso terapéutico , Resultado del TratamientoRESUMEN
PURPOSE: This retrospective cohort study aimed to investigate the effect of minimally invasive cochlear implantation (CI) on the vestibular function (VF) and residual hearing (RH) as well as their relationship in pediatric recipients before and after surgery. METHODS: Twenty-four pediatric patients with preoperative low frequency residual hearing (LFRH) (250 or 500 Hz ≤ 80 dB HL) who underwent minimally invasive CI were enrolled. Pure-tone thresholds, the cervical/ocular vestibular-evoked myogenic potential (cVEMP/oVEMP), and video head impulse test (vHIT) were all evaluated in the 24 pediatric patients with preoperative normal VF before and at 1 and 12 months after surgery. The relationship between changes in hearing and VF was analyzed preoperatively and at 1 and 12 months postoperatively. RESULTS: There were no significant differences on VF preservation and hearing preservation (HP) at both 1 and 12 months post-CI (p > 0.05). At 1 month post-CI, the correlations of the variations in vestibulo-ocular reflex (VOR) gains of horizontal semicircular canal (HSC) and posterior semicircular canal (PSC) and the shift in 250 Hz threshold were negatively correlated (r = - 0.41, p = 0.04 and r = - 0.43, p = 0.04, respectively). At 12 months post-CI, the shift in 250 Hz threshold negatively correlated to the variations in VOR gain of superior semicircular canal (SSC) (r = - 0.43, p = 0.04); the HP positively correlated to the variation in oVEMP-amplitude ratio (AR) (r = 0.41, p = 0.04). CONCLUSION: Our study confirmed that there were partial correlations between VF preservation and HP both in the short- and long-terms after atraumatic CI surgery, especially with the 250 Hz threshold. Regarding the variation of PSC function, the correlation with hearing status was variable with time after atraumatic CI surgery. Minimally invasive techniques for HP are successful and effective for the preservation of VF in pediatric patients both in the short- and long-terms.
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Implantación Coclear , Procedimientos Quirúrgicos Mínimamente Invasivos , Potenciales Vestibulares Miogénicos Evocados , Humanos , Implantación Coclear/métodos , Femenino , Masculino , Estudios Retrospectivos , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Preescolar , Niño , Potenciales Vestibulares Miogénicos Evocados/fisiología , Reflejo Vestibuloocular/fisiología , Audiometría de Tonos Puros , Resultado del Tratamiento , Audición/fisiología , Pruebas de Función Vestibular , Prueba de Impulso Cefálico/métodos , Vestíbulo del Laberinto/fisiopatología , Vestíbulo del Laberinto/cirugía , LactanteRESUMEN
OBJECTIVE: We investigated correlations among clinical features, degree of inner ear endolymphatic hydrops (EH), and hippocampal volume (HV) in different stages of Meniere's disease (MD). METHODS: From February 2021 to April 2022, clinical data were collected from 99 patients (39 males, 60 females, mean age: 50.4 ± 10.0 [range: 26-69] years) with unilateral MD admitted to the Department of Vertigo Disease of Shandong ENT Hospital. The left and right ears were affected in 64 and 35 patients, respectively. There were 50 and 49 cases in early (Stages 1, 2) and late stages (Stages 3, 4), respectively. Fifty healthy participants were included as controls. Audiovestibular function test results, EH grading using gadolinium-enhanced magnetic resonance imaging (MRI), and HV determined on MRI were analyzed for patients at different stages of MD. RESULTS: Between-group comparisons of early and late MD revealed significant differences in the disease course, vestibular function (VF), degree of EH, and HV. There were no significant between-group differences based on age, sex, affected side, subjective degree of dizziness, hospital anxiety, or depression. Mean HV in patients with early-stage MD was correlated with the canal paresis value of the caloric test and pure tone hearing threshold, HV in late-stage patients was correlated with vestibular EH. CONCLUSION: Patients with late-stage MD exhibited severe auditory and VF impairments, increased EH, and atrophy of the HV. More advanced disease was associated with greater vestibular damage and degree of EH. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:410-418, 2024.
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Hidropesía Endolinfática , Enfermedad de Meniere , Vestíbulo del Laberinto , Masculino , Femenino , Humanos , Adulto , Persona de Mediana Edad , Enfermedad de Meniere/complicaciones , Enfermedad de Meniere/diagnóstico por imagen , Hidropesía Endolinfática/diagnóstico por imagen , Hidropesía Endolinfática/complicaciones , Vestíbulo del Laberinto/diagnóstico por imagen , Vértigo/complicaciones , Atrofia/complicaciones , Hipocampo/diagnóstico por imagen , Hipocampo/patología , Imagen por Resonancia Magnética/métodosRESUMEN
The etiology of Ménière's disease (MD) remains controversial. Allergies are potential extrinsic factors that, in conjunction with underlying intrinsic factors, may cause MD. The link between allergies and MD was first described in 1923. For nearly a century, studies have demonstrated a possible link between allergies and MD, even though a causal relationship has not been definitively determined. Previous reviews have mainly focused on clinical epidemiology studies of patients. In this review, we shed light on the association between allergies and MD not only in terms of its epidemiology, but also from an immunology, pathophysiology, and immunotherapy perspective in both patients and animal models. Patients with MD tend to have a high risk of comorbid allergies or an allergy history, showing positive allergy immunology characteristics. Other MD-related diseases, such as migraine, may also interact with allergies. Allergy mediators such as IgE may worsen the symptoms of MD. Deposits of IgE in the vestibular end organs indicate the ability of the inner ear to participate in immune reactions. Allergic challenges can induce vertigo in animals and humans. Anti-allergy therapy plays a positive role in patients with MD and animal models of endolymphatic hydrops.
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Hidropesía Endolinfática , Hipersensibilidad , Enfermedad de Meniere , Animales , Humanos , Enfermedad de Meniere/epidemiología , Enfermedad de Meniere/etiología , Enfermedad de Meniere/terapia , Hidropesía Endolinfática/diagnóstico , Hidropesía Endolinfática/etiología , Hipersensibilidad/epidemiología , Hipersensibilidad/terapia , Hipersensibilidad/complicaciones , Inmunoterapia , Inmunoglobulina ERESUMEN
OBJECTIVE: A special presentation of foreign body granuloma originating from the lateral process of the malleus (FBGLP) was noted in the absence of a history of foreign body entry into the external auditory canal (EAC). This study reported the clinical features, pathology, and prognosis of patients with FBGLP. DESIGN: Retrospective study. SETTING: Shandong Provincial ENT Hospital. PATIENTS: Nineteen pediatric patients (age, 1-10 yr) with FBGLP. INTERVENTIONS: Clinical data were collected from January 2018 to January 2022. MAIN OUTCOME MEASURES: Clinicopathologic characteristics of the patients were analyzed. RESULTS: All patients had an acute course, and were within 3 months of ineffective medical treatment. The most common symptoms were suppurative (57.9%) and hemorrhagic (42.1%) otorrhea. FBGLP imaging examinations demonstrated a soft mass blocking the EAC without bone destruction and occasionally concomitant effusion in the middle ear. The most common pathologic findings were foreign body granuloma (94.7%,18/19), granulation tissue (73.7%, 14/19), keratotic precipitate (73.7%, 14/19), calcium deposition (63.2%, 12/19), hair shafts (47.4%, 9/19), cholesterol crystals (5, 26.3%), and hemosiderin (15.8%, 3/19). Foreign body granuloma and granulation tissue showed higher expression levels of CD68 and cleaved caspase-3 than did the normal tympanic mucosa, whereas Ki-67 levels were similarly low in all tissues. The patients were followed up for 3 months to 4 years without recurrence. CONCLUSION: FBGLP is caused by endogenous foreign particles in the ear. We recommend the trans-external auditory meatus approach for FBGLP surgical excision, as this shows promising outcomes.
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Granuloma de Cuerpo Extraño , Martillo , Niño , Humanos , Lactante , Preescolar , Estudios Retrospectivos , Granuloma de Cuerpo Extraño/cirugía , Granuloma de Cuerpo Extraño/complicaciones , Conducto Auditivo Externo/diagnóstico por imagen , Conducto Auditivo Externo/cirugía , Oído MedioRESUMEN
OBJECTIVES: The study goals were to compare the long-term efficacy of semicircular canal plugging (SCP) with labyrinthectomy in the treatment of advanced Meniere's disease (MD). STUDY DESIGN: A retrospective study. SETTING: Single tertiary medical center. METHODS: A total of 116 MD patients (TSCP group of 90; labyrinthectomy group of 26) with complete medical documents in Shandong Provincial ENT Hospital, from March 2017 to March 2019 were retrospectively analyzed, including a battery of auditory and vestibular function tests, recovery time from imbalance and function level scores (FLS). RESULTS: The total control rate of vertigo in the TSCP group was 96.7% (87/90). The rate of hearing loss was 23.3% (21/90). The control rate of vertigo in the labyrinthectomy group was 100% (26/26). All patients lost their auditory function after labyrinthectomy with a 100% hearing loss rate. There was no significant difference in the vertigo control rate between the two groups (P > 0.05). The hearing loss rate in the TSCP group was significantly lower than that in the labyrinthectomy group (P < 0.00). The median time recovered from imbalance was 15 days in TSCP group and 21 days in labyrinthectomy group, which is significantly different (P < 0.05). There was no significant difference in the FLS between the two groups (P > 0.05). CONCLUSIONS: Compared to labyrinthectomy, TSCP can preserve hearing at a high probability; meanwhile, otolith organ function preservation benefits patients from faster vestibular compensation. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:3178-3184, 2023.
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Sordera , Pérdida Auditiva , Enfermedad de Meniere , Humanos , Enfermedad de Meniere/cirugía , Estudios Retrospectivos , Canales Semicirculares/cirugía , Vértigo/etiología , Vértigo/cirugíaRESUMEN
Cochlear implant (CI) provision is the most effective clinical treatment to restore hearing performance in individuals with profound sensorineural hearing loss (SNHL). It has been successful in providing improved speech perception outcomes, especially in quiet environments. However, speech perception performance within complex environments, lexical tone recognition, and music perception have been shown to only improve with newer fine structure coding strategies or related techniques. Therefore, the methods used to assess hearing performance in noisy environments, lexical tone recognition, and music perception are of vital importance. These assessments must reflect the postoperative outcomes and also provide guidance for the programming, rehabilitation, and application of new coding strategies. In this study, hearing performance in simple and complex situations was evaluated before and after upgrading to a fine structure strategy. The participants were a cohort of Mandarin-speaking adolescents, who were experienced CI users. The comprehensive clinical workflow involved assessments of speech in quiet conditions, speech in noisy conditions, lexical tone recognition, and music perception. This battery of tests is explained in detail, from the coding strategy to the test methods, including the test process, environment, device, material, and order. The details that require special attention are discussed, such as the position of the participants, the angle of the loudspeaker, the intensity of the sound, the noise type, the practice test, and the way of answering questions. Each test step, method, and material for speech, lexical tone, and music perception is presented in detail. Finally, the clinical results are discussed.
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Implantación Coclear , Implantes Cocleares , Sordera , Pérdida Auditiva Sensorineural , Percepción del Habla , Humanos , Adolescente , Sordera/rehabilitación , Sordera/cirugía , Pérdida Auditiva Sensorineural/cirugía , AudiciónRESUMEN
Ménière's disease, a multifactorial disorder of the inner ear, is characterized by severe vertigo episodes and hearing loss. Although the role of immune responses in Ménière's disease has been proposed, the precise mechanisms remain undefined. Here, we show that downregulation of serum/glucocorticoid-inducible kinase 1 is associated with activation of NLRP3 inflammasome in vestibular-resident macrophage-like cells from Ménière's disease patients. Serum/glucocorticoid-inducible kinase 1 depletion markedly enhances IL-1ß production which leads to the damage of inner ear hair cells and vestibular nerve. Mechanistically, serum/glucocorticoid-inducible kinase 1 binds to the PYD domain of NLRP3 and phosphorylates it at Serine 5, thereby interfering inflammasome assembly. Sgk-/- mice show aggravated audiovestibular symptoms and enhanced inflammasome activation in lipopolysaccharide-induced endolymphatic hydrops model, which is ameliorated by blocking NLRP3. Pharmacological inhibition of serum/glucocorticoid-inducible kinase 1 increases the disease severity in vivo. Our studies demonstrate that serum/glucocorticoid-inducible kinase 1 functions as a physiologic inhibitor of NLRP3 inflammasome activation and maintains inner ear immune homeostasis, reciprocally participating in models of Ménière's disease pathogenesis.
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Hidropesía Endolinfática , Enfermedad de Meniere , Animales , Ratones , Glucocorticoides , Inflamasomas , Proteína con Dominio Pirina 3 de la Familia NLR , MacrófagosRESUMEN
It is challenging to program children with cochlear nerve deficiency (CND) due to limited auditory and speech abilities or concurrent neurological deficits. Electrically evoked compound action potential (ECAP) thresholds have been widely used by many audiologists to help cochlear implant programming for children who cannot cooperate with behavioral testing. However, the relationship between ECAP thresholds and behavioral levels of cochlear nerve in children with CND remains unclear. This study aimed to investigate how well ECAP thresholds are related to behavioral thresholds in the MAP for children with CND. This study included 29 children with CND who underwent cochlear implantation. For each participant, ECAP thresholds and behavioral T-levels were measured at three electrode locations across the electrode array post-activation. The relationship between ECAP thresholds and behavioral T-levels was analyzed using Pearson's correlation coefficient. The results showed that ECAP thresholds were significantly correlated with behavioral T-levels at the basal, middle, and apical electrodes. ECAP thresholds were equal to or higher than the behavioral T-levels for all tested electrodes, and fell within MAP's dynamic range for approximately 90% of the tested electrodes. Moreover, the contour of the ECAP thresholds was similar to the contour of T-levels across electrodes for most participants. ECAP thresholds can help audiologists select stimulation levels more efficiently for children with CND who cannot provide sufficient behavioral response.
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Implantación Coclear , Implantes Cocleares , Humanos , Niño , Potenciales de Acción , Umbral Auditivo/fisiología , Potenciales Evocados/fisiología , Implantación Coclear/métodos , Nervio Coclear/fisiología , Potenciales Evocados Auditivos/fisiología , Estimulación EléctricaRESUMEN
There is currently no satisfactory treatment for otolith dysfunctions. Here, we propose a novel surgical method, vestibule plugging (VP), and the results confirm its effectiveness and safety in treatment of otolith dysfunction. Laryngoscope, 133:1734-1736, 2023.
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Membrana Otolítica , Vestíbulo del Laberinto , HumanosRESUMEN
Otitis media with effusion (OME), also known as secretory otitis media, is a common condition in otorhinolaryngology. The main manifestations include middle ear effusion and conductive hearing loss. Recently, increasing attention has been paid to the etiology of OME, wherein immune dysfunction is one important pathogenic mechanism. However, it is unknown whether changes in surfactant protein A (SPA) secretion affect the phagocytic activity of macrophages in the Eustachian tube, thereby altering pathogen clearance, during the pathogenesis of OME. In our study, an OME animal model was established and evaluated. Differences in SPA levels in Eustachian tube lavage fluid between the experimental and control groups were analyzed. Cell-based experiments revealed that SPA decreased the expression of CD64 and SYK and inhibited phagocytosis by RAW264.7 cells. By using flow cytometry and immunofluorescence, we confirmed that macrophage phagocytosis decreased with increasing SPA levels. Finally, we concluded that SPA affects macrophage function and plays a role in the occurrence and development of OME.
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Otitis Media con Derrame , Animales , Proteína A Asociada a Surfactante Pulmonar , Macrófagos , Modelos Animales de Enfermedad , FagocitosisRESUMEN
OBJECTIVES: In this study, we aimed to (1) review the long-term outcomes of cochlear implantation in children with cochlear nerve aplasia and (2) compare the development of their auditory and speech abilities to children with normal-sized cochlear nerves. DESIGN: This is a retrospective case-control study. Patients who underwent unilateral cochlear implant (CI) surgery in a tertiary referral center from September 2012 to December 2018 were reviewed. The study group included 55 children with cochlear nerve aplasia diagnosed using preoperative images. The control group included 35 children with normal-sized cochlear nerves. The control group did not differ from the study group in terms of age at implantation, pre-implantation auditory and speech abilities, or the electrode array type. Cochlear implantation outcomes were assessed using a test battery, including the Categories of Auditory Performance (CAP) score, the Speech Intelligibility Rating (SIR) score, behavioral audiometry, and closed- or open-set speech recognition tests. The development of auditory and speech abilities was compared between the two groups using Generalized Linear Mixed-effect Models. RESULTS: The mean duration of CI usage was 4.5 years (SD = 1.5, range = 2.0 to 9.5) in the study group. The CAP scores, SIR scores, and aided hearing thresholds improved significantly post-implantation in the study group, but were significantly poorer than those in the control group. Generalized Linear Mixed-effect Models showed that the development of CAP and SIR scores was significantly slower in the study group than in the control group. Overall, 27 (49%) children with cochlear nerve aplasia had some degree of open-set speech perception skills, but the monosyllabic and bisyllabic word recognition rates were significantly lower than those in the control group. CONCLUSION: For children with cochlear nerve aplasia, auditory perception and speech intelligibility continued to improve in the long-term follow-up, but this progress was significantly slower than in children with normal-sized cochlear nerves. Most children with cochlear nerve aplasia could obtain the ability of common phrase perception and understanding simple spoken language with consistent CI usage and auditory rehabilitation.
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Implantación Coclear , Implantes Cocleares , Sordera , Percepción del Habla , Niño , Humanos , Lactante , Implantación Coclear/métodos , Estudios Retrospectivos , Estudios de Casos y Controles , Pruebas Auditivas , Percepción del Habla/fisiología , Inteligibilidad del Habla/fisiología , Nervio Coclear/anomalías , Resultado del Tratamiento , Sordera/cirugíaRESUMEN
Objective: To explore the long-term efficacy and safety of dexamethasone treatment via tympanic antrum catheterization (TAC) in intractable Meniere's disease (MD). Methods: In this retrospective analysis, 60 unilateral intractable MD patients treated with TAC in our hospital from January 2020 to August 2020 were followed for 2 years. Fifty patients who underwent endolymphatic sac decompression (ESD) and 50 patients who accepted intratympanic steroids (ITS) were established as the control groups. Vertigo control, hearing level, tinnitus, aural fullness and functional level were assessed during the study. Results: The effective vertigo control rate of intractable MD patients with TAC treatment was 76.7% (46/60) after 2 years follow-up, with a complete control rate of 58.3% (35/60) and a substantial control rate of 18.3% (11/60). The vertigo control rate of TAC was comparable to that of ESD (χ 2 = 0.313, p > 0.05), and significantly higher than that of ITS (χ 2 = 4.380, p < 0.05). The hearing loss rate of these patients was 10.8% (4/37), which was not significantly different from the control groups (χ 2 = 2.452, p > 0.05). The tinnitus improvement rate of patients with TAC was 56.7% (34/60), which was significantly higher than that of patients with ESD (χ 2 =11.962, p < 0.001) and ITS (χ 2 =15.278, p < 0.001). The aural fullness improvement rate in the TAC group was 56.7% (34/60), which was significantly higher than that in the ESD (χ 2 = 11.962, p < 0.001) and ITS groups (χ 2 = 5.635, p < 0.05). The functional level improvement rate in the TAC group was 71.7% (43/60), which was much higher than that in the ITS group (χ 2 = 17.256, p < 0.001), but there was no significant difference between TAC and ESD (χ 2 = 0.410, p > 0.05). No patients had complications or adverse reactions following TAC treatment. Conclusion: Dexamethasone treatment via TAC can effectively control vertigo attacks and improve related symptoms of intractable MD patients, providing valuable new insights into the treatment of MD.
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Background: Migraine plays an important role in some subgroups of children with recurrent vertigo. Moreover, the migraine component varies from definite to possibly absent as defined in this spectrum of three disorders-vestibular migraine of childhood (VMC), probable VMC (pVMC), and recurrent vertigo of childhood (RVC). However, studies on the sensory organization of balance control in these three disorders are rare. Objective: To explore the balance control of children with RVC, VMC, and pVMC, when the three sensory systems are challenged. Method: A retrospective analysis was performed on 125 children with VMC (18 female and 15 male; aged 11.64 ± 2.74), pVMC (10 female and eight male; aged 11.78 ± 2.51), and RVC (32 female and 42 male; aged 11.10 ± 2.60). All children in each subtype were divided into groups of children aged ≤ 12 years old and 13-17 years old. Vestibular examination screening and assessment for postural control using the six conditions of the sensory organization test (SOT) were performed. The three primary outcome measures were: equilibrium score (ES), strategy score (SS), and sensory analysis score of the SOT. Results: Equilibrium score under six different conditions and composite score increased with age (all P-values < 0.05). The somatosensory and visual scores also improved with growing (P-values < 0.05). However, vestibular scores did not increase significantly with age as the other senses did (P > 0.05). In the children ≤ 12 year-old group, children with VMC had a significantly higher visual preference score than those with pVMC and RVC (P < 0.05). There was an effect of age on the horizontal HIT. Ocular vestibular evoked myogenic potential (oVEMP), cervical vestibular evoked myogenic potential (cVEMP), and unilateral weakness (UW) values showed no significant difference among three diseases. Conclusion: Compared with patients at the age of 13-17 years old and with RVC and pVMC (both ≤ 12 years old), children with VMC had a higher degree of reliance on visual signals to maintain their balance and a poorer central integration of peripheral information before reaching 12 years of age. In addition, vision may predominate by weakening vestibular function based on visuo-vestibular interactions. It must be noted that peripheral vestibular examinations could not distinguish the three disease subtypes.
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Background: Cochlear implantation (CI) is an effective and successful method of treating individuals with severe-to-profound sensorineural hearing loss (SNHL). Coupled with it's great clinical effectiveness, there is a risk of vestibular damage. With recent advances in surgical approach, modified electrode arrays and other surgical techniques, the potential of hearing preservation (HP) has emerged, in order to preserve the inner ear function. These techniques may also lead to less vestibular damage. However, a systematic study on this at different follow-ups after CI surgery has not been documented before. Aims: To investigate changes of vestibular function systematically in recipients at short and long follow-ups after a minimally invasive CI surgery. Methods: In this retrospective study, 72 patients (72 ears) with minimally invasive CI were recruited. All participants selected had bilateral SNHL and pre-operative residual hearing (RH) and underwent unilateral CI. They were treated to comprehensive care. All patients underwent vestibular function tests 5 days prior to CI. During the post-operative period, follow-up tests were performed at 1, 3, 6, 9, and 12 months. The contemporaneous results of caloric, cervical vestibular-evoked myogenic potential (cVEMP), ocular vestibular-evoked myogenic potential (oVEMP), and video head impulse (vHIT) tests were followed together longitudinally. Results: On the implanted side, the percent fail rate of caloric test was significantly higher than that of vHIT at 1, 3, and 9 months post-operatively (p < 0.05); the percent fail rate of oVEMP was higher than vHIT of superior semicircular canal (SSC), posterior semicircular canal (PSC), or horizontal semicircular canal (HSC) at 1, 3, and 9 months (p < 0.05); at 3 and 9 months, the percent fail rate of cVEMP was higher than that of SSC and PSC (p < 0.05). There were no significant differences in the percent fail rates among all tests at 6 and 12 months post-CI (p > 0.05). The percent fail rates showed decreased trends in caloric (p = 0.319) and HSC tested by vHIT (p = 0.328) from 1-3 to 6-12 months post-operatively. There was no significant difference in cVEMP between 1-3 and 6-12 months (p = 0.597). No significant differences on percent fail rates of cVEMP and oVEMP between short- and long-terms post-CI were found in the same subjects (p > 0.05). Before surgery, the abnormal cVEMP and oVEMP response rates were both lower in patients with enlarged vestibular aqueduct (EVA) than patients with a normal cochlea (p = 0.001, 0.018, respectively). Conclusion: The short- and long-term impacts on the vestibular function from minimally invasive CI surgery was explored. Most of the vestibular functions can be preserved with no damage discrepancy among the otolith and three semicircular canal functions at 12 months post-CI. Interestingly, a similar pattern of changes in vestibular function was found during the early and the later stages of recovery after surgery.
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OBJECTIVES: To evaluate the clinical characteristics and prognostic factors of simultaneous and sequential bilateral sudden sensorineural hearing loss (Si-BSSNHL and Se-BSSNHL, respectively). DESIGN: Retrospective case-control study. SETTING: A single tertiary referral centre. PARTICIPANTS: Patients diagnosed with unilateral sudden sensorineural hearing loss (USSNHL), Si-BSSNHL, or Se-BSSNHL between September 2018 and November 2019. MAIN OUTCOME MEASURES: Demographic and clinical characteristics, audiological features, laboratory results and hearing recovery were analysed for intergroup comparisons. Prognostic factors for BSSNHL were analysed using univariate and multivariate logistic analyses between the overall and no-recovery groups. RESULTS: Compared to the USSNHL group, a larger final pure-tone average (PTA) (H = 38.0 and 53.8, respectively, both adjusted p-value (p adj) <.05), lower hearing gain (H = -70.8 and - 74.6, respectively, both p adj <.001) and higher homocysteine levels (H = 46.8, 58.8, respectively, both p adj <.05) were observed in the Si-BSSNHL and Se-BSSNHL groups, while the rate of positive vestibular tests and proportion of tinnitus were lower in the Se-BSSNHL group (χ2 = 8.5 and 38.1, respectively, both p adj <0.05). The USSNHL group showed a significant difference in the degree of deafness and therapeutic outcome in the Se-BSSNHL and Si-BSSNHL groups, respectively (χ2 = 12.4, p adj <.05; χ2 = 13.6; p adj <.05). Hypertension (95% confidence interval, 1.014-28.623, p < .05) and onset days (95% confidence interval, 0.007-0.626, p < .05) were associated with the therapeutic effects of BSSNHL. CONCLUSIONS: Higher homocysteine levels in BSSNHL may implicate microvascular disorders as a causative factor of BSSNHL. Hypertension and onset days were associated with the prognosis of BSSNHL.
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Pérdida Auditiva Sensorineural , Pérdida Auditiva Súbita , Hipertensión , Audiometría de Tonos Puros/métodos , Estudios de Casos y Controles , Pérdida Auditiva Bilateral/diagnóstico , Pérdida Auditiva Súbita/diagnóstico , Pérdida Auditiva Súbita/tratamiento farmacológico , Homocisteína/uso terapéutico , Humanos , Pronóstico , Estudios RetrospectivosRESUMEN
This study aimed to assess the function of the cochlear nerve using electrically evoked compound action potentials (ECAPs) for children with cochlear implants who were diagnosed with cochlear nerve aplasia and to analyze the correlation between preimplantation imaging results and ECAP responses. Thirty-five children diagnosed with cochlear nerve aplasia based on magnetic resonance imaging (MRI) were included. Preimplantation MRI and high-resolution computed tomography (HRCT) images were reconstructed, and the width of the bone cochlear nerve canal (BCNC), the diameter of the vestibulocochlear nerve (VCN), and the diameter of the facial nerve (FN) were measured. ECAP input/output (I/O) functions were measured at three electrode locations along the electrode array for each participant. The relationship between ECAP responses (including ECAP threshold, ECAP maximum amplitude, and slope of ECAP I/O function) and sizes of the BCNC and VCN was analyzed using Pearson's correlation coefficients. Our analysis revealed that ECAP responses varied greatly among individual participants. Overall, ECAP thresholds gradually increased, while maximum amplitudes and ECAP I/O function slopes gradually decreased, as the electrode location moved from the basal to the apical direction in the cochlea. ECAP responses exhibited no significant correlations with BCNC width or VCN diameter. The ratio of the VCN to FN diameters was significantly correlated with the slope of the ECAP I/O function and the maximum amplitude. BCNC width could not predict the function of the cochlear nerve. Compared with the absolute size of the VCN, the size of the VCN relative to the FN may represent an indicator for predicting the functional status of the cochlear nerve in children diagnosed with cochlear nerve aplasia based on imaging results.